近视盘视网膜毛细血管瘤的临床特点及随访观察

Clinical features and long-term follow-ups of juxtapapillary retinal capillary hemangioma

目的观察近视盘视网膜毛细血管瘤(RCH)的临床特征及治疗随访效果。方法回顾性病例系列研究。2007年1月至2019年12月于北京协和医院眼科检查确诊且随访时间>12个月的近视盘RCH(JRCH)患者14例15只眼纳入研究。患者中,男性7例7只眼,女性7例8只眼;平均年龄(29.8±12.7)岁。孤立型JRCH 6例,均为单眼患者,其中合并单个周边部RCH 1例;合并von Hippel-Lindau病8例,其中双眼RCH患者5例。15只眼中,合并周边部RCH 6只眼。单纯观察3只眼,行单纯光动力疗法(PDT)或PDT联合其他治疗12只眼。平均随访时间55.2个月。分析其临床特点及长期治疗效果。结果初诊时,15只眼中,视力<0.1者3只眼,0.1~0.4者5只眼,>0.5者7只眼。内生型、固着外生型JRCH分别为12(80%,12/15)、3(20%,3/15)只眼。瘤体大小1/4~4个视盘直径(DD);合并黄斑水肿、视网膜前膜、黄斑裂孔分别为11、5、1只眼。瘤体直径<1 DD者3只眼,初诊时视力为0.6~1.0,未行治疗。随访时间12个月者2只眼,120个月者1只眼。末次随访时,视力保持稳定,瘤体未增大。瘤体直径1.0~2.5 DD者6只眼,初诊时视力0.06~0.6;伴视网膜渗出。单纯PDT治疗5只眼,PDT联合抗血管内皮生长因子(VEGF)药物治疗1只眼。随访时间12~84个月。末次随访时,视力提高或稳定5只眼,下降1只眼。合并周边部RCH者6只眼,其中同时合并视网膜脱离4只眼、视网膜前膜2只眼、玻璃体积血1只眼,初诊时视力0.02~0.6。均行PDT联合经瞳孔温热疗法、巩膜外放液、玻璃体切割手术治疗。随访时间12~132个月。末次随访时,视力均下降。结论JRCH多于青壮年出现症状,双眼发病常见于von Hippel-Lindau病患者;瘤体可引起视网膜渗出、黄斑水肿、视网膜前膜和黄斑裂孔,造成视力缓慢下降。PDT和(或)联合抗VEGF药物治疗中等大小瘤体且伴有视网膜渗出者,可长期保持视力稳定;瘤体较大或合并视网膜脱离等严重并发症患者,治疗效果差。

Objective To evaluate the clinical features and follow-up effect of treatment for retinal capillary hemangioma(RCH)of myopic disc.Methods Retrospective case series studies.A total of 14 patients(15 eyes)diagnosed with juxtapapillary RCH(JRCH)at Peking Union Medical College Hospital and followed up for more than 12 months from January 2007 to December 2019 were included.Among these patients,there were 7 males(7 eyes)and 7 females(8 eyes)with the average age of 29.8±12.7 years.There were 6 isolated JRCH cases,all of which were monocular patients,including 1 case with a single peripheral RCH;8 cases with von Hippel-Lindau disease,including 5 cases of binocular RCH.Out of 15 eyes,6 eyes were merged with peripheral RCH.Three eyes were simply observed,and 12 eyes were treated with photodynamic therapy(PDT)alone or PDT combined with other treatments.The average follow-up time was 55.2 months.Its clinical characteristics and long-term treatment effects were analyzed.Results At the first visit,among the 15 eyes,3 eyes had visual acuity<0.1,5 eyes were 0.1-0.4,and 7 eyes were>0.5.The endogenous and fixed exogenous JRCH were 12(80%,12/15)and 3(20%,3/15)eyes,respectively.The size of the tumor was 1/4 to 4 optic disc diameters(DD);the combined macular edema,epiretinal membrane,and macular hole were 11,5,and 1 eyes,respectively.There were 3 eyes with tumor diameter less than 1 DD.The visual acuity at the first diagnosis was 0.6-1.0,and no treatment was given.The follow-up time was 12 months in 2 eyes and 120 months in 1 eye.At the last follow-up,his vision remained stable and the tumor did not enlarge.Six eyes with tumor diameter ranging from 1.0 to 2.5 DD,visual acuity was 0.06 to 0.6 at first diagnosis,accompanied by retinal exudation.5 eyes were treated with PDT alone,and 1 eye was treated with PDT combined with anti-vascular endothelial growth factor drugs.The follow-up time was 12 to 84 months.At the last follow-up,the visual acuity improved or stabilized in 5 eyes and decreased in 1 eye.There were 6 eyes with peripheral RCH,including 4 eyes with retinal detachment,2 eyes with epiretinal membrane,and 1 eye with vitreous hemorrhage.The visual acuity at the first visit was 0.02-0.6.All patients were treated with PDT combined with transpupillary thermotherapy,extrascleral drainage,and vitrectomy.The follow-up time was 12 to 132 months.At the last follow-up,all eyesight decreased.Conclusions Visual decrease cause by JRCH often occurs at young adults.Bilateral JRCH are more common in patients with von Hippel-Lindau's disease.The tumor can cause retinal exudation,macular edema,epiretinal membrane and macular hole,resulting in gradual vision loss.PDT and/or combined anti-vascular endothelial growth factor drugs for medium sized JRCH with retinal exudation can maintain long-term vision stability.Patients with large JRCH or severe complications such as retinal detachment tend to have poor prognosis.